Mitochondrial medicine: A metabolic perspective on the pathology of oxidative phosphorylation disorders

The final steps in the production of adenosine triphosphate (ATP) in mitochondria are executed by a series of multisubunit complexes and electron carriers, which together constitute the oxidative phosphorylation (OXPHOS) system. OXPHOS is under dual genetic control, with communication between the nu...

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Bibliographic Details
Published in:Cell metabolism 2006-01, Vol.3 (1), p.9-13
Main Authors: Smeitink, Jan A., Zeviani, Massimo, Turnbull, Douglass M., Jacobs, Howard T.
Format: Article
Language:English
Subjects:
Animals
Humans
Mitochondria - metabolism
Mitochondria - pathology
Mitochondrial Diseases - drug therapy
Mitochondrial Diseases - metabolism
Mitochondrial Diseases - pathology
Oxidative Phosphorylation
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Summary:The final steps in the production of adenosine triphosphate (ATP) in mitochondria are executed by a series of multisubunit complexes and electron carriers, which together constitute the oxidative phosphorylation (OXPHOS) system. OXPHOS is under dual genetic control, with communication between the nuclear and mitochondrial genomes essential for optimal assembly and function of the system. We describe the current understanding of the metabolic consequences of pathological OXPHOS defects, based on analyses of patients and of genetically engineered model systems. Understanding the metabolic consequences of OXPHOS disease is of key importance for elucidating pathogenic mechanisms, guiding diagnosis and developing therapies.
ISSN:1550-4131
1932-7420
DOI:10.1016/j.cmet.2005.12.001